About Me

My mother was murdered by what I call corporate and political homicide i.e. FOR PROFIT! she died from a rare phenotype of CJD i.e. the Heidenhain Variant of Creutzfeldt Jakob Disease i.e. sporadic, simply meaning from unknown route and source. I have simply been trying to validate her death DOD 12/14/97 with the truth. There is a route, and there is a source. There are many here in the USA. WE must make CJD and all human TSE, of all age groups 'reportable' Nationally and Internationally, with a written CJD questionnaire asking real questions pertaining to route and source of this agent. Friendly fire has the potential to play a huge role in the continued transmission of this agent via the medical, dental, and surgical arena. We must not flounder any longer. ...TSS

Introduction: Bovine Spongiform Encephalopathy (BSE) is the single animal
prion disease reputed to be zoonotic, inducing variant of Creutzfeldt-Jakob
Disease (vCJD) in man, and therefore strongly conditioned the protective
measures. Among different sources of animal prion diseases, we show here that
after more than ten years of incubation, intracerebral injection of a sheep
scrapie isolate can induce a prion disease in cynomolgus macaque, a relevant
model of human situation towards several prion strains. Neuropathological
studies showed classical and uncommon data.

Material and method: The cynomolgus macaque was intracerebrally exposed to
a classical scrapie isolate issued from a naturally infected sheep flock. Upon
onset of clinical signs, euthanasia was performed for ethical reasons. Classical
methods of biochemistry and neuropathology were used.

Results: The three elements of the triad were present:

spongiosis was predominant in the cortex, the striatum, the cerebellum.
Neuronal loss and gliosis were moderate.

The notable data were the following

(i) the brain was small, the atrophy involved mostly the temporal lobe in
which axonal loss was histologically demonstrated

(ii) the spongiosis of the Purkinje cells was so intense that most of them
were destroyed

(iii) there was a neuronal loss and a massive gliosis of the dorsomedialis
nucleus of the thalamus

(iv) iron deposits were present in the lenticular nucleus. PrPres heavily
distributed in the cortex, the basal ganglia and the cerebellum consisted in
synaptic deposits and aggregates. Western Blot exhibited a type 1 PrPres in all
parts of the brain.

Conclusion: We described here the successful transmission of a scrapie
prion disease to a non-human primate after an extended incubation period,
leading to a fatal, non-relapsing neurological disease with all the features of
a prion disease. The cerebral lesional profile we observed was original in
comparison to other animal prion diseases (c-BSE, L-type BSE, TME) we previously
experimentally transmitted in this model.

Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant
Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie
prions remains unknown. Mice genetically engineered to overexpress the human
​prion protein (tgHu) have emerged as highly relevant models for gauging the
capacity of prions to transmit to humans. These models can propagate human
prions without any apparent transmission barrier and have been used used to
confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie
prions transmit to several tgHu mice models with an efficiency comparable to
that of cattle BSE. The serial transmission of different scrapie isolates in
these mice led to the propagation of prions that are phenotypically identical to
those causing sporadic CJD (sCJD) in humans. These results demonstrate that
scrapie prions have a zoonotic potential and raise new questions about the
possible link between animal and human prions.

5. A positive result from a chimpanzee challenged severly would likely
create alarm in some circles even if the result could not be interpreted for
man. I have a view that all these agents could be transmitted provided a large
enough dose by appropriate routes was given and the animals kept long enough.
Until the mechanisms of the species barrier are more clearly understood it might
be best to retain that hypothesis.

What if you can catch old-fashioned CJD by eating meat from a sheep
infected with scrapie?

28 Mar 01 Most doctors believe that sCJD is caused by a prion protein
deforming by chance into a killer. But Singeltary thinks otherwise. He is one of
a number of campaigners who say that some sCJD, like the variant CJD related to
BSE, is caused by eating meat from infected animals. Their suspicions have
focused on sheep carrying scrapie, a BSE-like disease that is widespread in
flocks across Europe and North America.

Now scientists in France have stumbled across new evidence that adds weight
to the campaigners' fears. To their complete surprise, the researchers found
that one strain of scrapie causes the same brain damage in mice as sCJD.

"This means we cannot rule out that at least some sCJD may be caused by
some strains of scrapie," says team member Jean-Philippe Deslys of the French
Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses,
south-west of Paris. Hans Kretschmar of the University of Göttingen, who
coordinates CJD surveillance in Germany, is so concerned by the findings that he
now wants to trawl back through past sCJD cases to see if any might have been
caused by eating infected mutton or lamb...

2001

Suspect symptoms

What if you can catch old-fashioned CJD by eating meat from a sheep
infected with scrapie?

28 Mar 01

Like lambs to the slaughter

31 March 2001

by Debora MacKenzie Magazine issue 2284.

FOUR years ago, Terry Singeltary watched his mother die horribly from a
degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary
was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded
an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.

Most doctors believe that sCJD is caused by a prion protein deforming by
chance into a killer. But Singeltary thinks otherwise. He is one of a number of
campaigners who say that some sCJD, like the variant CJD related to BSE, is
caused by eating meat from infected animals. Their suspicions have focused on
sheep carrying scrapie, a BSE-like disease that is widespread in flocks across
Europe and North America.

Now scientists in France have stumbled across new evidence that adds weight
to the campaigners' fears. To their complete surprise, the researchers found
that one strain of scrapie causes the same brain damage in mice as sCJD.

"This means we cannot rule out that at least some sCJD may be caused by
some strains of scrapie," says team member Jean-Philippe Deslys of the French
Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses,
south-west of Paris. Hans Kretschmar of the University of Göttingen, who
coordinates CJD surveillance in Germany, is so concerned by the findings that he
now wants to trawl back through past sCJD cases to see if any might have been
caused by eating infected mutton or lamb.

Scrapie has been around for centuries and until now there has been no
evidence that it poses a risk to human health. But if the French finding means
that scrapie can cause sCJD in people, countries around the world may have
overlooked a CJD crisis to rival that caused by BSE.

Deslys and colleagues were originally studying vCJD, not sCJD. They
injected the brains of macaque monkeys with brain from BSE cattle, and from
French and British vCJD patients. The brain damage and clinical symptoms in the
monkeys were the same for all three. Mice injected with the original sets of
brain tissue or with infected monkey brain also developed the same
symptoms.

As a control experiment, the team also injected mice with brain tissue from
people and animals with other prion diseases: a French case of sCJD; a French
patient who caught sCJD from human-derived growth hormone; sheep with a French
strain of scrapie; and mice carrying a prion derived from an American scrapie
strain. As expected, they all affected the brain in a different way from BSE and
vCJD. But while the American strain of scrapie caused different damage from
sCJD, the French strain produced exactly the same pathology.

"The main evidence that scrapie does not affect humans has been
epidemiology," says Moira Bruce of the neuropathogenesis unit of the Institute
for Animal Health in Edinburgh, who was a member of the same team as Deslys.
"You see about the same incidence of the disease everywhere, whether or not
there are many sheep, and in countries such as New Zealand with no scrapie." In
the only previous comparisons of sCJD and scrapie in mice, Bruce found they were
dissimilar.

But there are more than 20 strains of scrapie, and six of sCJD. "You would
not necessarily see a relationship between the two with epidemiology if only
some strains affect only some people," says Deslys. Bruce is cautious about the
mouse results, but agrees they require further investigation. Other trials of
scrapie and sCJD in mice, she says, are in progress.

People can have three different genetic variations of the human prion
protein, and each type of protein can fold up two different ways. Kretschmar has
found that these six combinations correspond to six clinical types of sCJD: each
type of normal prion produces a particular pathology when it spontaneously
deforms to produce sCJD.

But if these proteins deform because of infection with a disease-causing
prion, the relationship between pathology and prion type should be different, as
it is in vCJD. "If we look at brain samples from sporadic CJD cases and find
some that do not fit the pattern," says Kretschmar, "that could mean they were
caused by infection."

There are 250 deaths per year from sCJD in the US, and a similar incidence
elsewhere. Singeltary and other US activists think that some of these people
died after eating contaminated meat or "nutritional" pills containing dried
animal brain. Governments will have a hard time facing activists like Singeltary
if it turns out that some sCJD isn't as spontaneous as doctors have
insisted.

Deslys's work on macaques also provides further proof that the human
disease vCJD is caused by BSE. And the experiments showed that vCJD is much more
virulent to primates than BSE, even when injected into the bloodstream rather
than the brain. This, says Deslys, means that there is an even bigger risk than
we thought that vCJD can be passed from one patient to another through
contaminated blood transfusions and surgical instruments.

In the room today we have a few delegates from the CFIA – Paul Mayers, Vice
President for Policy and Programs for the CFIA ainsi que Dr. Martine Dubuc,
Vice-présidente de la direction des sciences et déléguée pour le Canada à
l'Organisation mondial de la santé animale. Thank you for joining us here
today.

We will begin today's technical briefing with an update on the
investigation into BSE in Alberta with a statement from the CFIA. We will then
open the phone lines up for a question and answer period. J'aimerais maintenant
céder la parole à Paul Mayers.

Paul Mayers: Thank you very much Guy. Good afternoon everyone and thank you
for calling in today. We would like to provide an update on the Canadian Food
Inspection Agency's investigation into bovine spongiform encephalopathy or BSE
in a beef cow from Alberta. First of all I'd like to remind you that no part of
the animal's carcass entered the human food or animal feed systems.

Canada's suite of internationally recognized safeguards effectively
protects the safety of food and animal feed. There is no risk to food safety.
The CFIA's investigation into BSE in Alberta has determined that the affected
cow was born in March 2009. We were also able to confirm the location of the
birth farm yesterday evening. We can also confirm that both the birth and index
farm are located in northern Alberta near Edmonton.

The index farm is in the municipality of Spruce Grove. At this time the
CFIA cannot release the municipality of the birth farm as the agency is still
engaged in an active investigation at this premise. The CFIA was able to trace
the animal to its original birth farm through the combination of reviewing the
records at the index farm as well as a tattoo in the ear of the animal.

Both the index farm and birth farm continue to cooperate with the CFIA
throughout this investigation. With regards to the birth farm, the CFIA is
continuing its investigation efforts focusing on a review of on farm animal and
feed records to verify if there are other animals that are of equivalent
risk.

The CFIA will determine the source of the feed used at the birth farm in
2009 and assess any potential risk factors in relation to the feed used. As a
precautionary measure, CFIA inspectors will review the records of the feed mills
from which the feeds were sourced to verify compliance with the enhanced feed
ban. The enhanced feed ban was put in place to accelerate Canada's progress
towards the reduction of this disease.

As this progression continues, the detection of a small number of cases
among the 30,000 samples tested yearly as part of our BSE surveillance program
is not unexpected. This has been the case for most countries that are managing
BSE. These cases are extremely rare in Canada. This has been the first case in
four years. The number of cases seen in Canada has been steadily
declining.

This is due to the strong measures Canada has in place. The enhanced feed
ban, the comprehensive program of surveillance and the removal and disposal of
specified risk materials serve to effectively protect public health and minimize
the potential for recycling infection in the cattle population. Bill C18,
currently before Parliament, includes provisions which will enhance the Feeds
Act and the Health of Animals Act providing further regulatory tools for the
CFIA in these types of situations.

The government of Canada is committed to protecting human and animal health
and takes the management of BSE very seriously. The case has been reported to
the World Organisation for Animal Health, also known as the OIE, in line with
Canada's international obligations and our commitment to transparency. Canada is
officially recognized under the OIE's science based system as a controlled BSE
risk country.

This status clearly acknowledges the effectiveness of Canada's
surveillance, mitigation and disease management measures and acknowledges the
work done by all levels of government, the cattle industry, veterinarians and
ranchers to effectively manage BSE in Canada. The OIE has stated that this one
case won't change this status.

Therefore we expect our trading partners on the basis of this status to not
make any changes to market access for Canadian beef. However, market access
decisions are for individual countries to make. South Korea has imposed a
temporary restriction on the importation of Canadian beef pending further
information on the confirmed BSE find. Indonesia has temporarily suspended
importation of non-edible by-products.

Again, the CFIA is strongly committed to protecting animal health. Our
investigation is underway and we are mobilizing all necessary resources to
address this situation. More information on BSE and the actions Canada has taken
to address the disease are available at the CFIA website at inspection.gc.ca.
Thank you very much.

you can’t believe what I got in the US Postal mail today. the wife would
not pick it up yesterday, because there was a $6.00 charge for a certified
letter from USDA Kevin Shea for about 5 pages. I went to the PO today, told the
girls in the back that if it’s an affidavit, a warrant, summons, I don’t want
it, send it back. but it was certified. scared me. but the curiosity got to me,
so i coughed up 6 bucks, and took a chance. low and behold, after my last appeal
to this decade plus old quest was turned down, even though I already had the
answer from another source, APHIS et al finally stumbled across those old mouse
bio-assays. they had them all along.

what the industry sent me first was better, because it had some of the good
stuff i.e. redacted.

this all started way back around the year 2,000, when in my opinion, the
USDA et al let these sheep in the USA from Belgium, when they should not have
because of atypical BSE in Belgium. I started asking for the these mouse
bio-assays back in or around 2003 or before, then I had to get official with
FOIA, because no one would answer my questions.

well, it’s February 20, 2015, over a decade later, and I don’t know how
many denials, here’s what was in the mail yesterday, February 19, 2015 ;

This letter is in response to the Freedom of Information Act (FOIA) appeal
that you submitted regarding FOIA request 07-566. Your appeal challenged the
APHIS FOIA Office's search for the "Mouse Bio-Assays" on the sheep imported from
Belgium. We apologize for the delayed response.

The APHIS FOIA Office received your appeal, on July 7, 2007 and assigned it
FOIA case number 2007-00030-A.

In response to your appeal, the APHIS FOIA Office performed a second search
of records responsive to your initial request. The Agency has since found four
(4) pages of responsive records for the "Mouse Bio-Assays" dated October
22,2009. Although these records postdate both your initial request and
subsequent appeal by approximately two years; we enclose them in the interest of
responsiveness to your request.

We now consider this appeal closed and will take no further action. If you
are dissatisfied with this decision, you have the right to judicial review in an
appropriate United States District Court in accordance with 5 U.S.C. 552,
(2)(4)(B).

Prior to seeking judicial review, you may contact the Office of Government
Information Services (OGlS). OGIS was created within the National Archives and
Records Administration when the Open Government Act of 2007 amended the FOIA.
OGIS provides mediation of FOIA disputes between appellants and federal
agencies. Participation in mediation does not affect your right to judicial
review. Contact information for OGIS can be found at
http:/www.archives.gov/ogis.

Sincerely,

Kevin Shea Administrator Enclosure

snip...end

the next 4 pages is exactly what I received from an industry source way
back on Saturday, February 27, 2010. see ;

This is the FINAL report for contract MPL-6197-7-37 The testing of the
Belgian (Vermont) sheep.

Background

Brain homogenate (10% in normal saline) from each case was inoculated
intracerebralty into panels of 20 Rlll and 20 Tg338 mice.

FT1294/0001 (Sample 4677) was inoculated into mice on the 14/12/06

FT1294/0011 (Sample 4703) was inoculated into mice on the 20/12/06

Method

The brain from each mouse was examined histologically for any evidence of
TSE-related vacuolation, and immunolabelled using anti-PrP antibody Rb486 as
described elsewhere1, All slide interpretation was undertaken blind with regard
to the clinical status of the mouse, or the source of the inoculum.

Final bioassay results

FT1294/0001 (Sample 4677)

Tg338 mice - All 20 mice are negative by histopathology, and
immunohistochemistry

Rlll mice - All 20 mice are negative negative by histopathology, and
immunohistochemistry

FT1294/0011 (Sample 4703)

Tg338 mice - All 20 mice are negative by histopathology, and
immunohistochemistry

Rlll mice - All 20 mice are negative by histopathology, and
immunohrstochernistry

The survival times for these mice can be seen in the figures below.
Additional data sets from positive and negative inocula (J Spiropoulos, pers.
comm.) have been included for comparison._

VLA is an Executive Agency of the Department for Environment, Food and
Rural Affairs (Defra)

snip... (2 pages of charts and graphs of survival and comparison of tg338
data NOT included here...TSS)

CONCLUSION

These mice have survived for long enough to have demonstrated the presence
of classical scrapie, atypical scrapie, or ovine BSE if any of these strains was
present in the inoculum.

Both samples are negative by bioassay.

Dr. Marion M Simmons

22nd October 2009

February 27, 2010, INVESTIGATION OF MAD SHEEP OF MAD RIVER VALLEY COMPLETE.
THEY WERE NOT INFECTED WITH ANY TSE. ...TSS

Greetings again BSE list members,

The investigation of the Mad Sheep of Mad River Valley may be complete now,
but, I still have questions.

PLEASE SEE MY FINAL FOIA HERE ;

Monday, September 1, 2008

RE-FOIA OF DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL
T.S.E. (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [No. 00-072-1]
September 1, 2008

Greetings again BSE-L members,

I had a pleasant surprise this past Saturday. I got an unexpected package
from O.I.G. on my old F.O.I.A. request, of the final test results of the
infamous mad sheep of mad river valley. IF you all remember, back on Thu, 24 Apr
2008 15:00:20 -0500 I wrote ;

Greetings,

With great disgust, I must report, that after years and years of wrangling
over the infamous mad sheep of mad river valley, I have failed in getting an
official answer via FOIA on the outcome of the TSE testing of those imported
Belgium sheep. The USA Government refuses to tell the public, exactly what the
testing outcome was, and in doing so, shows just how corrupt this administration
has been. and the excuse given in their answer to my final appeal, which they
have now officially denied, was bizarre to say the least ;

"I am denying your FOIA appeal. This is the final agency decision. You may
seek judicial review of this decision in the United States district court for
the judicial district in which you reside or have your principal place of
business or in the District of Columbia, pursuant to 5 U.S.C. &
552(a)(4)(B)."

FOIA OF DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL
T.S.E. (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES [Docket No.
00-072-1] ...snip...end...TSS

NOW, out of the wild blue, AFTER them telling me they denied my FOIA appeal
for the final time, any further action would have to be judicial review in the
United States district court, I get 25+ pages, a lot of redacted names, etc, but
this is the first time they sent me anything about this in the 6 years of
waiting for my FOIA request. IT will take me a long time to get this online due
to the fact you cannot hardly read it, very poor quality and eligibility of
text. BUT, the just of it is, somebody (REDACTED) screwed those tests up. I will
work to get all the data online next week or so, but it is odd how much they
were concerned for human and animal health from an atypical scrapie of foreign
origin back then, but yet when we document it here in the USA, you don't hear a
word about it. it's a completely different story.

IN SHORT ;

August 15, 2000

OIG case # NY-3399-56 REDACTED, VT

''Enclosed is OIG's notification that they have scheduled an investigation
of the following individual. REDACTED is alleged to have provided possibly
inaccurate test results involving diseased sheep. However, because the results
were determined to be inconclusive, no actual violation was actually
committed.''

snip...

IN SHORT ;

August 15, 2000

OIG case # NY-3399-56 REDACTED, VT

''Enclosed is OIG's notification that they have scheduled an investigation
of the following individual. REDACTED is alleged to have provided possibly
inaccurate test results involving diseased sheep. However, because the results
were determined to be inconclusive, no actual violation was actually
committed.''

snip...

[only bush et al could have interpreted it that way. don't all criminals
wish this is the way the system worked. ...tss]

JULY, 28, 2000

Case Opening Memorandum

snip...

An investigation regarding the subject identified below will be conduced
and a report submitted at the conclusion of the investigation. If you have or
should later receive additional information concerning this matter, please
forward it to this office.

If you believe that administrative action should be taken before all
criminal and other legal matters are completed, please coordinate that action
with this office in order not to jeopardize the ongoing investigation.

The fact that this subject is under investigation should not be discussed
with anyone who does not have a need to know and all inquiries on the
investigation should be referred to the office of Inspector General.

snip...end

FOR OFFICIAL USE ONLY FEBRUARY 7, 2002

SUBJECT OIG CASE NY-3399-56 REDACTED VT HEALTH/SANITATION VIOLATION

TO: William Buisch, Regional Director Eastern Region, VS Raleigh, NC

Enclosed is the official investigation report on REDACTED. If you will
recall, REDACTED is alleged to have provided possible inaccurate test results
involving diseased sheep.

OIG is closing their file upon issuance of the Report of Investigation
(copy enclosed). We are, therefore, also closing our case file.

REDACTED

Resource Management Systems and Evaluation Staff

Enclosure

cc:

REDACTED IES, Riverdale, MD (w/cy of incoming)

APHIS:RMSES: REDACTED 2/7/02 "NY-3399-56-REDACTED Closure''

END...TSS

NOW, the question is, who screwed those test up, and was it done on
purpose, just to cover someone's ass for letting those sheep in here in the
first place ???

WHICH tests were compromised, one of them, all of them, and, can we trust
the outcome of any of these test under the circumstances here ???

i.e.

"It is significant that four of the sheep which first tested positive on
REDACTED Western blot tests, thereby providing the type of confirmation the
plaintiffs argue is lacking on the current record."

UNDER what circumstances were these test compromised ???

MY basic, simple question, was not answered in layman term, i.e. exactly
what strain of TSE did those sheep have ???

IS this the best we can do ???

>>>"REDACTED is alleged to have provided possibly inaccurate test
results involving diseased sheep. However, because the results were determined
to be inconclusive, no actual violation was actually committed.''<<<

*** FINAL REPORT OF THE TESTING OF THE BELGIAN (VERMONT) SHEEP February 27,
2010 IN SHORT ; August 15, 2000 OIG case # NY-3399-56 REDACTED, VT ''Enclosed is
OIG's notification that they have scheduled an investigation of the following
individual. REDACTED is alleged to have provided possibly inaccurate test
results involving diseased sheep. However, because the results were determined
to be inconclusive, no actual violation was actually committed.''

FINAL REPORT OF THE TESTING OF THE BELGIAN (VERMONT) SHEEP February 27,
2010

I respectfully request the final results of the mouse bio-assays test that
were to have supposedly began 2+ years late, 5 years ago, on the imported sheep
from Belgium ?

WHAT happened to the test results and MOUSE BIO-ASSAYS of those imported
sheep from Belgium that were confiscated and slaughtered from the Faillace's,
what sort of TSE did these animals have ?

WERE they atypical scrapie, BSE, and or typical scrapie ?

HOW much longer will you refuse to give us this information ? and for what
reason ?

WHY is it that the Farm of the Mad Sheep of Mad River Valley were
quarantined for 5 years, but none of these farms from Texas and Alabama with
Atypical TSE in the Bovine, they have not been quarantined for 5 years, why not,
with the real risk of BSE to sheep, whom is to say this was not BSE ?

UNITED STATES DEPARTMENT OF AGRICULTURE OFFICE OF INSPECTOR GENERAL
WASHINGTON D.C. 20250

DEC 28, 2007

Mr. Terry S. Singeltary, Sr. P.O. Box 42 Bacliff, Texas 77518

Subject: FOIA Appeal-Log No. 08-00034 (No. 07-00060)

Dear Mr. Singeltary:

This is in response to your December 3, 2007, Freedom of Information Act
(FOIA), 5 U.S.C. & 552, appeal of the November 20, 2007, decision of Ms.
Deirdre MacNeil, FOIA/Privacy Act (PA) Attorney, Office of Inspector General
(OIG), Department of Agriculture (USDA). As explained below, your FOIA appeal is
denied.

As background, on March 1, 2007, you requested the "final results of the
TSE Mouse-bioassays of those Atypical TSE in the Vermont Sheep." FOIA requires
the release of agency records except where one or more of the nine enumerated
exceptions apply. On November 20, 2007, Ms. MacNeil responded to your request by
sending you seven pages from Hotline files PS-3340-0024, which was responsive to
your request. Ms. MacNeil withheld identifying information pursuant to
Exceptions 6 and 7(C) of the FOIA. See 5. U.S.C.& 552(b)(6) and (7)(C). On
December 3, 2007, you appealed Ms. MacNeils decision.

12-3-07

To The Honorable Inspector General USDA,

I respectfully "APPEAL" the decision to withhold information I requested
under the F.O.I.A. About the final results of the T.S.E. Mouse-bioassays of the
Atypical T.S.E. in the Vermont Sheep imported from Belgium and later confiscated
and slaughtered under a "Extra Ordinary Declaration of Emergency due to Atypical
T.S.E. in U.S.A. sheep.

Log Number 07-00060 FOIA 07-566

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518

Exemption 6 permits the Government to withhold information about
individuals in "personnel and medical files and similar files the disclosure of
which would constitute a clearly unwarranted invasion of personal privacy." 5
U.S.C. & 552 (b)(6). To warrant protection under Exemption 6, information
must first meet a threshold requirement by falling within the category of
personnel and medical files and similar files. Id. Information fits into a
"similar file" if it contains information regarding a particular individual. See
United States Dept of State V. Washington Post Co., 456, 601-02 (1982). The
threshold is met in this case, as the memorandum contains information regarding
particular named individuals.

Exemption 7(C) protects from disclosure law enforcement information, the
disclosure of which "could reasonably be expected to constitute and unwarranted
invasion of personal privacy." 5 U.S.C. & 552(b)(7)(C. Under Exemption 7(C),
it has been held that a protectible privacy interest exists in the identities of
investigative agents. See Senate of

Mr. Terry S. Singeltary, Sr. Page 2

Puerto Rico v. United States Dep't of Justice, 823 F.2d 574, 588-89 (D.C.
Cir. 1987); Nishnic v. United States Dep't of Justice, 671 F. Supp. 776, 789
(D.D.C. 1987). Such a privacy interest exists in this case, as the withheld
information contains the identities, including names and identifying
information, of investigative agents in the memorandum.

Once it is determined that a privacy interest exists, Exemptions 6 and
7(C), of FOIA require a balancing of interests between the public interest
served by disclosure and an individual's right to privacy. See, e.g., Senate of
Puerto Rico, 823 F.2d at 587; Dep't of the Air Force v. Rose, 425 U.S. 352, 372
(1976). Determination of whether disclosure is warranted turns not upon the
particular purpose for which the document is requested, but upon the nature of
the requested document and its relationship to the central purpose of FOIA,
which is to "open agency action to the light of public scrutiny." United States
Dep't of Justice v. Reporters Comm. for Freedom of the Press, 489 U.S. 749,
772-73 (1989) (quoting Rose, 425 U.S. at 372). I have determined that the
release of the withheld information, of investigative agents in the memorandum,
would not serve the public interest. Therefore, I am denying your appeal with
respect to the withholdings pursuant to Exemptions 6 and 7(C).

In addition to appealing the exemptions pursuant to 6 and 7(C), you appear
to take issue with USDA's Animal $ Plant Health Inspection Service's (APHIS)
response to your FOIA requests with APHIS. You may contact APHIS regarding the
status of any such requests by contacting Mr. Garfield Daley, Acting FOIA
Officer, at (301)734-5273, 4700 River Road, Unit 50, Riverdale, MD,
20737-1232

For these reasons, I am denying your FOIA appeal. This is the final agency
decision. You may seek judicial review of this decision in the United States
district court for the judicial district in which you reside or have your
principal place of business or in the District of Columbia, pursuant to 5 U.S.C.
& 552(a)(4)(B).

Sincerely,

David R. Gray

FOR

Phyllis K. Fong

Inspector General

=======END...TSS...4.24.08=======

----- Original Message -----

From: Terry S. Singeltary Sr.

To: Boyd.Rutherford@usda.gov

Sent: Sunday, February 25, 2007 12:35 PM

Subject: FOIA REQUEST FOR ATYPICAL TSE INFORMATION ON VERMONT SHEEP

Greetings USDA,

I respectfully request the final results of the mouse bio-assays test that
were to have supposedly began 2+ years late, 5 years ago, on the imported sheep
from Belgium ?

WHAT happened to the test results and MOUSE BIO-ASSAYS of those imported
sheep from Belgium that were confiscated and slaughtered from the Faillace's,
what sort of TSE did these animals have ?

WERE they atypical scrapie, BSE, and or typical scrapie ?

HOW much longer will you refuse to give us this information ? and for what
reason ?

WHY is it that the Farm of the Mad Sheep of Mad River Valley were
quarantined for 5 years, but none of these farms from Texas and Alabama with
Atypical TSE in the Bovine, they have not been quarantined for 5 years,why not,
with the real risk of BSE to sheep, whom is to say this was not BSE ?

FURTHERMORE, I respectfully request up front, that any fees for this FOIA
be wavered due to the fact this information should be free to the public and is
in the best interest for the public to have these final results, no financial
gain from this FOIA information is to be made either. ...

Thank You,

kind regards,

Terry S. Singeltary Sr.

P.O. Box 42

Bacliff, Texas USA 77518

Imported

Belgium/Netherlands

Sheep Test Results

Background

Factsheet

Veterinary Services April 2002

APHIS

snip...

Additional tests will be conducted to determine exactly what TSE the
animals have BSE or scrapie. These tests involve the use of bioassays that
consist of injecting mice with tissue from the infected animals

Page 15 of 98

8/3/2006

and waiting for them to develop disease. This testing may take at least 2
to 3 years to complete.

Additional tests will be conducted to determine exactly what TSE the
animals have BSE or scrapie. These tests involve the use of bioassays that
consist of injecting mice with tissue from the infected animals

Page 15 of 98

8/3/2006

and waiting for them to develop disease. This testing may take at least 2
to 3 years to complete.

######## Bovine Spongiform Encephalopathy
#########Greetings
list members,Thought I
should let the list know that Dr. Detwiler kindly replied to myquestion
about the delayed 'atypical' TSE testing in the Vermont sheep andtried to
explain what caused the delay. If I interpreted it correctly,seems it was
the fault of the U.K. ;--------
Original Message --------Subject: SheepDate: Sat, 12 Jun 2004 14:26:04
EDTFrom: LAVET22@aol.comTo: flounder@wt.netMr.
Singeltary.I hope this
finds you well. As you are aware I left the USDA lastyear. I can only update
you on the sheep before that time. Contact wasestablished with the UK on
doing the bioassay studies. They agreed.However, we were prioritized after
their own needs, hence the delay. Iam aware that there are now additional
labs in Europe running the mousebioassay strain typing. You will have to
contact USDA for further word.Linda
Detwiler=========My reply to
Dr. Detwiler;-------- Original Message --------Subject: Re:
SheepDate: Sat, 12 Jun 2004 13:53:57 -0500From: "Terry S. Singeltary
Sr." To: LAVET22@aol.comReferences:
<54 .2bd2ac1e.2dfca4bc="" aol.com="">54>hello Dr.
Detwiler,thanks for
your kind reply.>
However, we were prioritized after their own needs, hence the
delay.not sure i
understand that?> You
will have to contact USDA for further word.already
done that, and there answer was;>5/20/04>>Dear
Mr. Singeltary,>>The Western blot tests on these animals were
completed in April of this>year. That means that we can begin the mouse
inoculations. To get the>results of the Western blot tests, you will need
to submit a Freedom of>Information Act request through our FOIA office.
The FAX number there is>301-734-5941.>>Have a nice
day,>>Jim Rogers>APHIS LPA>and with my
previous attempts for information via the FOIA throughthis administration
(as you are probably very well aware of) they haveall been ignored/refused.
so any further attempts would be fruitless i amsure.thanks
anyway...kindest
regards,TerryLAVET22@aol.com wrote:> Mr.
Singeltary.snip...TSSTerry S.
Singeltary Sr. wrote:>
######## Bovine Spongiform Encephalopathy > #########>>
Greetings Dr. Detwiler,>> glad to see you are still with us, you
had become very silent lately.> hope you are enjoying semi
retirement.>> recently, i inquired through the BSE-L and via USDA official
about> those Vermont sheep via belgium which there was an
Extraordinary> Declaration of Emergency declared here in the USA due
to> atypical scrapie. The thread is;>> Confiscation of
Sheep in Vermont and testing results ? Thu, 20 May 2004> 12:10:03 -0500
"Terry S. Singeltary Sr." Bovine> Spongiform Encephalopathy
BSE-L>>>>> Imported>>
Belgium/Netherlands>> Sheep Test Results>>
Background>> Factsheet>> Veterinary Services April
2002>> APHIS>>>>
snip...>>> Additional tests will be conducted to
determine>> exactly what TSE the animals have BSE or
scrapie.>> These tests involve the use of bioassays that
consist>> of injecting mice with tissue from the infected
animals>> and waiting for them to develop disease. This
testing>> may take at least 2 to 3 years to
complete.>>>> http://www.aphis.usda.gov/lpa/pubs/fsheet_faq_notice/fs_ahvtsheeptr.pdf>> DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL
T.S.E.> (PRION DISEASE) OF FOREIGN ORIGIN IN THE UNITED
STATES>> http://frwebgate.access.gpo.gov/cgi-bin/getdoc.cgi?dbname=2000_register&docid=fr20jy00-32>>>
DECLARATION OF EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E> (PRION DISEASE) OF
FOREIGN ORIGIN IN THE UNITED STATES [2]>> http://frwebgate.access.gpo.gov/cgi-bin/getdoc.cgi?dbname=2000_register&docid=fr20jy00-31>>>
or if those old urls dont work, go here;>> DECLARATION OF
EXTRAORDINARY EMERGENCY BECAUSE OF AN ATYPICAL T.S.E> (PRION
DISEASE) OF FOREIGN ORIGIN IN THE UNITED STATES> - Terry S. Singeltary
Sr. 7/20/00 (0)>>> I was told that
;>>> -------- Original Message --------> Subject:
Re: hello Dr. Sutton...question please...scrapie...TSS> Date: Thu, 20 May
2004 14:36:09 -0400> From: Jim.D.Rogers@aphis.usda.gov> To:
flounder@wt.net>>>> Dear Mr.
Singeltary,>> The Western blot tests on these animals were
completed in April of this> year. That means that we can begin the mouse
inoculations. To get the> results of the Western blot tests, you will
need to submit a Freedom of> Information Act request through our FOIA
office. The FAX number there is> 301-734-5941.>> Have a
nice day,>> Jim Rogers> APHIS LPA>
=========>>> Dr. Detwiler, my question is, why have these
very important test been> delayed for so long when we were told they were
to have been started> some 2+ years ago?>> who made this
call to delay these very important test and why ?>> thank
you,> with kindest regards,>> Terry>>>
Linda Detwiler wrote:>>> ######## Bovine Spongiform
Encephalopathy >> #########>>>> I m responding to
Roland's post about my quote in the article by Steve>> Mitchell. I
spent a fair amount of time on the phone with Mr.>> Mitchell on more
than>> one occasion. The quote was one aspect of our conversation.
Even>> the quote>> included "probably". I explained about
proper location and sampling>> condition of the brain. I also added in
our conversation that the>> best methodology is>> to utilize
both a test for PrP as well as histopathology when examining>> brains
from cattle with CNS disease. This is why as early as>> 1993-94 the
USDA>> began using IHC in its TSE testing regime at the National
Veterinary>> Services>> Laboratory. However, utilizing only
a PrP test eliminates the>> possibility of>> diagnosing
another neurologic disease.>>>> All of the tests have
advantages and disadvantages. For example, I>> have been>>
to a number of laboratories in Europe and watched as technicians
took>> the>> test samples from the brain stem. They sample
hundreds per night.>> If they get>> distracted the may take
the sample lateral, or rostral to the>> obex. If this>>
animal was in an earlier stage of disease, there may be a very small>>
amount of>> PrPsc and limited to a single location in the brain. If
that sample>> missed>> the obex, the sample would be
negative and life goes on. With any>> test using>> a
homogenate I am not aware that there is any cross check for
location.>>>> With IHC, the pathologist can determine
location, however it too has>> drawbacks in regard to sample
condition. There is no perfect test.>> There are>>
limitations to the tests themselves and there are limitations to all>>
of the aspects>> of collection. Hence utilizing multiple tests
especially for CNS>> cases is>> prudent. It is also prudent
to examine other locations of the brain>> in the event>> a
disease changes or something new emerges.>>>>>>
Linda Detwiler>>>> #########
http://mailhost-alt.rz.uni-karlsruhe.de/warc/bse-l.html>>
##########>>>>>>>> #########
http://mailhost-alt.rz.uni-karlsruhe.de/warc/bse-l.html>
##########>#########
http://mailhost-alt.rz.uni-karlsruhe.de/warc/bse-l.html
##########

Saturday, February 27, 2010

*** FINAL REPORT OF THE TESTING OF THE BELGIAN (VERMONT) SHEEP

February 27, 2010 IN SHORT ;

August 15, 2000

OIG case # NY-3399-56 REDACTED, VT

''Enclosed is OIG's notification that they have scheduled an investigation
of the following individual. REDACTED is alleged to have provided possibly
inaccurate test results involving diseased sheep. However, because the results
were determined to be inconclusive, no actual violation was actually
committed.''

7. WHY is it that the Farm of the Mad Sheep of Mad River Valley were
quarantined for 5 years, but none of these farms from Texas and Alabama with
Atypical TSE in the Bovine, they have not been quarantined for 5 years, why not,
with the real risk of BSE to sheep, whom is to say this was not BSE ?

SOME DISTURBING TSE DATA FROM BELGIUM ;

Increased incidence of sporadic Creutzfeldt-Jakob disease in the age groups
between 70 and 90 years in Belgium

(7) Laboratory of Neurobiology, BBI, UA, CDE, Universiteitsplein 1, B-2610
Wilrijk, Belgium Received: 28 October 2005 Accepted: 28 March 2006 Published
online: 12 July 2006 Abstract From 1998 a prospective surveillance study of
Creutzfeldt-Jakob disease (CJD) has been initiated in Belgium. In addition to
epidemiological data, information on cerebrospinal fluid biomarkers, prion
protein gene and brain neuropathology was collected. From 1-1-1998 to
31-12-2004, 188 patients were referred to the surveillance system. In 85
patients a ‘definite’ diagnosis of sporadic CJD (sCJD) could be made, whereas 26
patients remained ‘probable’. We further identified two unrelated patients with
an E200K mutation, and two patients with a seven octapeptide repeat insertion in
one family. In one patient a familial history was noted but genetic analysis was
not performed. In 72 patients different final diagnoses were made, Alzheimer’s
disease being the most frequent (N = 20). The demographic parameters of the
Belgian population were similar to those observed in the rest of Europe. We did
notice a significantly increased age-specific incidence (‰>‰6/106/year) of
sCJD patients between 70 and 90 years old in the period 2002– 2004 compared to
1998–2001 and retrospectively obtained data (1990–1997, p < 0.01). We
undertook a detailed clinical and biochemical analysis to investigate this
increase but could not identify any reason other than an increased vigilance for
the diagnosis.

In conclusion, our study identified that in the past sCJD may have been
underestimated in patients over age 70 although these patients are both
clinically and neurobiochemically similar to the general sCJD phenotype.
Keywords Diagnosis - Epidemiology - Prion disease - Transmissible spongiform
encephalopathy

For many years, researchers believed that only one bovine spongiform
encephalopathy (BSE) strain existed, in contrast to the many different scrapie
strains found. However, only very recently reports emerged about unconventional
BSE strains seen in Italy, France, and Japan. The present case describes an
atypical strain of BSE in Belgium in a 64-month old East-Flemish cow with an
electrophoretic profile and other features similar to those described in
Japan.

INTRODUCTION

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are a
group of fatal neurodegenerative diseases including sheep and goat scrapie,
bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease (CJD) in
humans. They are characterized by the accumulation of an abnormal protein,
called PrPsc, which is formed post translationally from the normal isoform
(PrPc).1,2 At present, the agent causing TSEs is still incompletely
characterized, although PrPsc is believed to be its major if not unique
constituent.3

Research in mice showed the existence of different scrapie strains.4,5
Scrapie strain discrimination is currently based on biologic typing in a panel
of inbred mice, using incubation time and brain pathology scoring as criteria.6
However, no large-scale studies of the molecular features of PrPsc have been
reported for bovine BSE to date. Till now, the BSE strain seemed to maintain
constant biologic and molecular properties even after experimental or accidental
passages into different species, such as mice, humans, primates, and sheep.7–10
However, very recently, variant forms of BSE have been reported in Japan, Italy,
and France.11-13 These forms were characterized by atypical histopathologic,
immunohistochemical, or biochemical phenotypes. The present case is the
description of the first atypical BSE case in Belgium.

MATERIALS AND METHODS

Since January 2001, all cattle older than 30 months are tested for TSE via
a rapid test (TeSeE-kit, Bio-Rad, Nazareth, Belgium) after EC regulation
999/2001.14,15 Samples positive according to the enzyme-linked immunosorbent
assay (ELISA) screening are further subjected to scrapie-associated fibrils
(SAF), histopathology, immunohistochemistry, and Western blot (WB) testing 16,17
at the National Reference Laboratory (NRL).

RESULTS

A positive ELISA sample from a 64-month-old East-Flemish cow or Belgian
white and red (Figure 1) was presented at the NRL for confirmation. The animal
was reported healthy before slaughter. The optical density (OD) titers at the
local laboratory were 2.324 and 2.116.16 The OD titers at the NRL were 0.953 and
0.708 (sample taken at the contralateral side of the first sampling side of the
obex region). The histopathology of the obex, pons, and midbrain

Page 19 of 98

8/3/2006

showed no spongiform changes; immunohistochemistry of the brainstem
revealed no signal of PrPsc accumulation typical for BSE; and SAF was negative.
However, WB analysis (Bovine WB, Bio-Rad, France; antibodies 12F10 and SAF60) of
the same homogenate that was prepared from the obex region for ELISA revealed a
small amount of PrPsc with an electrophoretic profile different from that of
typical BSE-associated PrPsc.18,19 The band on the gel of the non-glycosylated
form of PrPsc of the present case clearly showed a lower migration pattern
compared with that of a typical BSE case (Figure 2).

DISCUSSION

For many years, researchers assumed that only one BSE strain existed.7–10
Only in the past months, reports of atypical BSE cases were announced.11–13 The
Japanese case11 describes a very young bull (23 months) characterized by the
absence of spongiform changes and PrPsc deposits immunohistochemically. The WB
analysis revealed an electrophoretic profile different from that of typical BSE,
characterized by low content of the di-glycosylated molecular form of PrPsc and
a faster migration of the nonglycosylated form of PrPsc. In Italy,12 two BSE
affected cattle with a previously unrecognized neuropathologic profile and PrPsc
type were seen. These cases were determined using a different staining pattern
on immunohistochemistry, a difference in size and glycoform ratio of PrPsc on
immunoblot and a difference in regional distribution of lesions. The two cases
in France13 showed variant molecular features with a different PrPsc
electrophoretic profile from other BSE cases, mainly characterized by a higher
molecular mass of the nonglycosylated PrPsc. The present case shows the most
similarities (ie, identical electrophoretic profile, only ELISA and WB positive
and histopathology and immunohistochemistry negative) with the Japanese case,11
although the cow in the Japanese case was only 23 months old, and the cow in
this case was 64 months old.

The fact that these strains were detected worldwide and in several breeds
suggest that there is no local or breed dependent feature involved. It could be
that the WB techniques have become more specific within the past year in the
detection of minor differences in di-, mono-, and nonglycosylated molecular
forms of PrPsc. Infection of cattle by scrapie could also be considered since
scrapie can be transmitted by direct contact between animals or through
environmental contamination.13

In conclusion, this Belgian case should be added to the list of atypical
BSE strains only very recently detected worldwide and may contribute to further
research studies about epidemiologic significance. Current continued research on
BSE would appear to reveal different BSE strains in analogy with the different
scrapie strains.

Comment #6: WHAT happened to the test results and MOUSE BIO-ASSAYS of those
imported sheep from Belgium that were confiscated and slaughtered from the
Faillace's, what sort of TSE did these animals have?

Response: It is not clear how the test results referred to in this comment
are relevant to the Harvard BSE Risk Assessment Update. Sheep were not
considered in the risk assessment.

Comment #7: WHY is it that the Farm of the Mad Sheep of Mad River Valley
were quarantined for 5 years, but none of these farms from Texas and Alabama
with Atypical TSE in the Bovine, they have not been quarantined for 5 years, why
not, with the real risk of BSE to sheep, whom is to say this was not BSE ?

Response: This comment pertains to policy. As such, it is not addressed
here.

THE
REST IS HISTORY, around 2005, what they were trying to stop at the Faillaces
front door, was then finally documented anyway, and since then, the Nor-98
‘foreign animal disease’ Winking smile , has spread from coast to coast in North
America.

***
The discovery of previously unrecognized prion diseases in both humans and
animals (i.e., Nor98 in small ruminants) demonstrates that the range of prion
diseases might be wider than expected and raises crucial questions about the
epidemiology and strain properties of these new forms. We are investigating this
latter issue by molecular and biological comparison of VPSPr, GSS and Nor98.

>BSE-suspected Vermont Sheep Get
Another>Reprieve, USDA Backs Off Slaughter
Order>>BURLINGTON, Vt., August 10 (United Press International) --
Two>flocks of exotic sheep scheduled for slaughter this week in
Vermont>because of fears some of the animals might be infected with a
form>of the deadly mad cow disease have won yet another
reprieve.>>The U.S. Department of Agriculture has temporarily
backed off plans>to seize and destroy the animals, the Burlington Free
Press reported>Thursday.>

Actually something else happened in the
interim:

Vermont sheep still grazing as lawyers reach deal

Thu, Aug 10, 2000 Reuters

BURLINGTON, Vt., Aug 10 (Reuters) - Lawyers for owners of Vermont
sheepsuspected of having a neurological ailment similar to mad cow
diseasestruck a deal with prosecutors on Thursday for a fuller court hearing
onthe animals' fate.

Under the agreement, lawyers for the farmers will forgo
afederalappeals case in return for a more detailed hearingUnder the
agreement, lawyers for the farmers will forgo afederalappeals case in return
for a more detailed hearing in a lower court, saidTom Amidon, who represents
sheep farmer Houghton Freeman."So the sheep are on the farm awhile," Amidon
said. He hoped a newhearing would allow for closer examination of claims by
the U.S.Agriculture Department, which wants to seize and eliminate the 350
sheepunder scrutiny as a health precaution.

U.S. District Judge Garvan Murtha ruled last week the
AgricultureDepartment could carry out its plan. But the farmers planned to
appealbefore Thursday's agreement changed the legal picture."We will
drop our appeal to the second circuit and in return the U.S.attorneys office
in Vermont will allow us to hold a fuller court hearing,"Amidon said.

Four sheep on Vermont farms near Warren, Vermont, tested positive
lastmonth for a disease known as TSE or transmissible
spongiformencephalopathy, according to USDA officials.

TSE can cause scrapie, a fatal disease in sheep. It is also part of
afamily of diseases that includes deadly BSE, also known as
bovinespongiform encephalopathy or mad cow disease.

No cases of BSE or mad cow disease have ever been found in the
UnitedStates, but the human form of the disease is blamed for 75 deaths
inBritain during the 1980s.

Breeding pairs for the two flocks of diary sheep came from Belgium
andthe Netherlands in the mid-90s.

Amidon said the Belgian government has sent a letter to the
USDArequesting the return of all 350 sheep and that Freeman had already
linedup a plane to transport them. Whether that will eventually occur
remains

SCIENTIFIC COMMITTEE OF THE BELGIAN FEDERAL AGENCY FOR THE SAFETY OF THE
FOOD CHAIN

Subject: RAPID ADVICE 17-2014 : Evaluation of the risk for public health
of casings in countries with a “negligible risk status for BSE” and on the risk
of modification of the list of specified risk materials (SRM) with regard to BSE

SCIENTIFIC COMMITTEE OF THE BELGIAN FEDERAL AGENCY FOR THE SAFETY OF THE
FOOD CHAIN

RAPID ADVICE 17-2014

Subject: Evaluation of the risk for public health of casings in
countries with a “negligible risk status for BSE” and on the risk of
modification of the list of specified risk materials (SRM) with regard to BSE
(Dossier SciCom 2014/22) Rapid advice approved by the Scientific Committee on
22nd October 2014.

Summary

The
Scientific Committee was asked to answer two questions in regard to a proposal
from the European Commission to no longer obligate Member States with a
negligible BSE risk status to remove and dispose the specified risk materials as
specified in Annex V to Regulation (EC) No 999/2001 of the European Parliament
and of the Council of 22 May 2001 laying down rules for the prevention, control
and eradication of certain transmissible spongiform encephalopathies. The aim of
this modification of the Regulation is to ensure that conditions for imports of
commodities from third countries are not more favorable than the conditions
applying to Member States with the same OIE BSE negligible risk status. More
specifically it was asked to the Scientific Committee:

-
If there is a difference in public health risk between the casings imported from
third countries with a “negligible risk status for BSE” and casings that come
from the 18 EU Member States with a “negligible risk status for BSE”?

-
If there is a significantly increased public health risk if, in the EU Member
States with a “negligible risk status for BSE”, the intestines are no longer
removed as SRM and if the other risk materials for BSE (the skull including the
brains and eyes, the spinal cord, the tonsils and the spine) are indeed
considered as SRM?

Due
to lack of availability of data on true prevalence and tissue infectivity of BSE
(classical as well as atypical BSE) the Scientific Committee was not able to
thoroughly investigate the questions.

The
Scientific Committee is of the opinion that, taking into consideration the
uncertainties in regard to the true prevalence of BSE (classical as well as
atypical BSE) in countries with a “negligible risk status for BSE” and given the
problems related with the early detection of asymptomatic BSE and given the
zoonotic significance of atypical BSE, that stopping with the routine removal of
specified risk materials during bovine slaughter will increase the risk for
public health.

2/14

The
Scientific Committee is not able to compare the public health risk of casings
from third countries and from the 18 EU Member States, both with a negligible
risk status for BSE, because of lack of data on true BSE prevalence and BSE
tissue infectivity (classical BSE and atypical BSE) in the considered
countries.

The
Scientific Committee is also not able to properly answer the second question if
there is a significantly increased public health risk if, in the EU Member
States with a “negligible risk status for BSE”, the intestines are no longer
removed as SRM due to lack of quantitative data on tissue infectivity of
different specified risk materials in slaughtered bovines in these countries.
There is also no information on tissue infectivity of atypical BSE cases. It is
known however that intestines are the portal of entry of prions and that they
are already infective before the prions reach the central nervous system.

The
final decision pertaining the need of removal of all or part of the specified
risk materials is a risk management decision and goes beyond the competencies of
the Scientific Committee.

In
conclusion the Scientific Committee is not able to answer this question with an
acceptable degree of uncertainty because of lack of data on true prevalence of
BSE (classical as well as atypical forms of BSE) in the considered countries. It
reiterates its concern regarding the import of certain animal products from
third countries with a ‘negligible BSE risk status’ as stated in rapid advice
SciCom 16-2013.

snip...

2.
Is there a significantly increased public health risk if, in the EU Member
States with a “negligible risk status for BSE”, the intestines are no longer
removed as SRM while the other risk materials for BSE (the skull including the
brains and eyes, the spinal cord, the tonsils and the spine) are indeed
considered as SRM?

Once again the Scientific Committee is not able to properly answer this
question because of lack of quantitative data on tissue infectivity of different
specified risk materials in slaughtered bovines in EU Member States with a
“negligible risk status for BSE”.

BSE
infected animals may enter undetected the food chain due to the low sensitivity
of the diagnostic tests. Further on the classical BSE agent accumulates from the
first months post exposure in particular segments of the bovine intestines and
persists till clinical onset. In addition no information is available about the
infectivity of tissues by the atypical BSE agent, especially in the
intestines.

If
intestines from cattle in EU Member States with a “negligible risk status for
BSE” are no longer removed as SRM and are allowed to enter the food chain the
public health risk will be increased. The degree of rise in risk level cannot be
determined. According to EFSA Journal 2014;12(2):3554, the TSEi model indicated
that the removal of the last four meters of the small intestine and of the
caecum from the food and feed chain would result in a major reduction of the
Classical BSE exposure risk associated with intestine and mesentery in
cattle.

Referring to its previous advice 16-2013 the Scientific Committee
repeats that stopping with the routine removal of all specified risk materials
during bovine slaughter will increase the risks of exposure of the population to
BSE because of the uncertainty related to the detection of BSE. This uncertainty
is the consequence of the long incubation period (especially in cases of
atypical BSE), the low sensitivity of the available diagnostic methods, the
apparent spontaneous nature of atypical BSE, the lack of a clear clinical
picture of atypical BSE cases and the reduction in number of tests in healthy
slaughtered animals.

The
final decision pertaining the need of removal of all or part of the specified
risk materials is a decision to be taken by the risk manager and goes beyond the
competencies of the Scientific Committee.

The
Scientific Committee is of the opinion that, taking into consideration the
uncertainties in regard to the true prevalence of BSE (including classical as
well asaAtypical BSE) in countries with a “negligible risk status for BSE” and
given the problems related with the early detection of asymptomatic BSE and
given the zoonotic character of atypical BSE, stopping with the routine removal
of all specified risk materials during bovine slaughter will increase the risk
for public health.

12/14

The
Scientific Committee is not able to compare the public health risk of casings
from third countries and from the 18 EU Member States both with a negligible
risk status for BSE because of lack of data on true BSE prevalence (classical
BSE and atypical BSE) in the considered countries.

The
Scientific Committee is not able to properly answer the question if there is a
significantly increased public health risk if, in the EU Member States with a
“negligible risk status for BSE”, the intestines are no longer removed as SRM
due to lack of quantitative data on tissue infectivity of different specified
risk materials in slaughtered bovines in these countries. There is also no
information on tissue infectivity by the agent of atypical BSE.

On
behalf of the Scientific Committee, The President Prof. Dr. E. Thiry (Sgd.)
Brussels, 06/11/2014

Background: Recently atypical forms of BSE have been described. Western
blot analyses showed that, in comparison to the classic BSE (C-type), they are
demonstrable by a higher or lower molecular weight of the unglycosylated PrPres.
They Viere thus named H-type and L-type BSE (L-type is also called BASE). In
addition they show a lower proportion of diglycosylated PrPres than C-type.
These emerging types represent different strains of BSE. They show unique
incubation periods and histological lesions. Such types have been described on
different continents. Indeed they might correspond to "sporadic" forms of BSE.
In 2004 we already described one L-type in Belgium.

Objective: We retrospectively analysed the bovines at least 7-year-old
in the Belgian archive of BSE ­diagnosed cattle in order to determine the
prevalence of the two types of atypical BSE in Belgium.

Methods: We analysed homogenates from 39 bovines of 93 months old in
median (min: 84, max: 181 months). The most recent one was diagnosed in 2006. We
used Western blot with a panel of anti-PrP antibodies (Ab). They detect
different regions of the PrP protein, from N-terminal to C-terminal: 12B2, 9A2,
Sha31. SAFB4, 94B4. Their combination is aimed at an efficient typing
diagnostic. We detected bound Ab with SuperSignal West Dura (Pierce) and
analysed PrPres, signals with an image-analysis software (Quantity One,
Bio-Rad).

Results: The results are still under analysis. We will detail the most
crucial characteristics for typing PrPres. These include 1) the apparent
molecular mass of the an-, mono- and diglycosylated bands, 2) the binding
affinity to the five Ab (e.g.12B2 for H-type), 3) the presence of a fourth
(unglycosylated) band and 4) the glycoprofile based on the relative proportions
of the visible bands.

Discussion: The emergence of atypical types of BSE is partially due to a
better knowledge of prion strains and more efficient diagnostic techniques. As
the area in the brain where the PrPres is deposited can differ drastically
between the types, it is essential to ascertain that the sampling techniques and
analyses are adapted to these new types. As these new strains seem more virulent
than classic types, they represent one of the next challenges in the field of
prions.

For
many years, researchers believed that only one bovine spongiform encephalopathy
(BSE) strain existed, in contrast to the many different scrapie strains found.
However, only very recently reports emerged about unconventional BSE strains
seen in Italy, France, and Japan. The present case describes an atypical strain
of BSE in Belgium in a 64-month-old East-Flemish cow with an electrophoretic
profile and other features similar to those described in Japan.

INTRODUCTION

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are
a group of fatal neurodegenerative diseases including sheep and goat scrapie,
bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease (CJD) in
humans. They are characterized by the accumulation of an abnormal protein,
called PrPsc, which is formed post-translationally from the normal isoform
(PrPc).1,2 At present, the agent causing TSEs is still incompletely
characterized, although PrPsc is believed to be its major if not unique
constituent.3

Research in mice showed the existence of different scrapie strains.4,5
Scrapie strain discrimination is currently based on biologic typing in a panel
of inbred mice, using incubation time and brain pathology scoring as criteria.6
However, no large-scale studies of the molecular features of PrPsc have been
reported for bovine BSE to date. Till now, the BSE strain seemed to maintain
constant biologic and molecular properties even after experimental or accidental
passages into different species, such as mice, humans, primates, and sheep.7-10
However, very recently, variant forms of BSE have been reported in Japan, Italy,
and France.11-13 These forms were characterized by atypical histopathologic,
immunohistochemical, or biochemical phenotypes. The present case is the
description of the first atypical BSE case in Belgium.

snip...

In
conclusion, this Belgian case should be added to the list of atypical BSE
strains only very recently detected worldwide and may contribute to further
research studies about epidemiologic significance. Current continued research on
BSE would appear to reveal different BSE strains in analogy with the different
scrapie strains.

For
many years, researchers believed that only one bovine spongiform encephalopathy
(BSE) strain existed, in contrast to the many different scrapie strains found.
However, only very recently reports emerged about unconventional BSE strains
seen in Italy, France, and Japan. The present case describes an atypical strain
of BSE in Belgium in a 64-month-old East-Flemish cow with an electrophoretic
profile and other features similar to those described in Japan.

INTRODUCTION

Transmissible spongiform encephalopathies (TSEs), or prion diseases, are
a group of fatal neurodegenerative diseases including sheep and goat scrapie,
bovine spongiform encephalopathy (BSE), and Creutzfeldt-Jakob disease (CJD) in
humans. They are characterized by the accumulation of an abnormal protein,
called PrPsc, which is formed post-translationally from the normal isoform
(PrPc).1,2 At present, the agent causing TSEs is still incompletely
characterized, although PrPsc is believed to be its major if not unique
constituent.3

Research in mice showed the existence of different scrapie strains.4,5
Scrapie strain discrimination is currently based on biologic typing in a panel
of inbred mice, using incubation time and brain pathology scoring as criteria.6
However, no large-scale studies of the molecular features of PrPsc have been
reported for bovine BSE to date. Till now, the BSE strain seemed to maintain
constant biologic and molecular properties even after experimental or accidental
passages into different species, such as mice, humans, primates, and sheep.7 10
However, very recently, variant forms of BSE have been reported in Japan, Italy,
and France.11-13 These forms were characterized by atypical histopathologic,
immunohistochemical, or biochemical phenotypes. The present case is the
description of the first atypical BSE case in Belgium.

MATERIALS AND METHODS

Since January 2001, all cattle older than 30 months are tested for TSE
via a rapid test (TeSeE-kit, Bio-Rad, Nazareth, Belgium) after EC regulation
999/2001.14,15 Samples positive according to the enzyme-linked immunosorbent
assay (ELISA) screening are further subjected to scrapie-associated fibrils
(SAF), histopathology, immunohistochemistry, and Western blot (WB) testing16,17
at the National Reference Laboratory (NRL).

RESULTS

A
positive ELISA sample from a 64-month-old East-Flemish cow or Belgian white and
red (Figure 1) was presented at the NRL for confirmation. The animal was
reported healthy before slaughter. The optical density (OD) titers at the local
laboratory were 2.324 and 2.116.16 The OD titers at the NRL were 0.953 and 0.708
(sample taken at the contralateral side of the first sampling side of the obex
region). The histopathology of the obex, pons, and midbrain showed no spongiform
changes; immunohistochemistry of the brainstem revealed no signal of PrPsc
accumulation typical for BSE; and SAF was negative. However, WB analysis (Bovine
WB, Bio-Rad, France; antibodies 12F10 and SAF60) of the same homogenate that was
prepared from the obex region for ELISA revealed a small amount of PrPsc with an
electrophoretic profile different from that of typical BSE-associated
PrPsc.18,19 The band on the gel of the non-glycosylated form of PrPsc of the
present case clearly showed a lower migration pattern compared with that of a
typical BSE case (Figure 2).

DISCUSSION

For
many years, researchers assumed that only one BSE strain existed.7 10 Only in
the past months, reports of atypical BSE cases were announced.11 13 The Japanese
case11 describes a very young bull (23 months) characterized by the absence of
spongiform changes and PrPsc deposits immunohistochemically. The WB analysis
revealed an electrophoretic profile different from that of typical BSE,
characterized by low content of the di-glycosylated molecular form of PrPsc and
a faster migration of the nonglycosylated form of PrPsc. In Italy,12 two BSE
affected cattle with a previously unrecognized neuropathologic profile and PrPsc
type were seen. These cases were determined using a different staining pattern
on immunohistochemistry, a difference in size and glycoform ratio of PrPsc on
immunoblot and a difference in regional distribution of lesions. The two cases
in France13 showed variant molecular features with a different PrPsc
electrophoretic profile from other BSE cases, mainly characterized by a higher
molecular mass of the nonglycosylated PrPsc. The present case shows the most
similarities (ie, identical electrophoretic profile, only ELISA and WB positive
and histopathology and immunohistochemistry negative) with the Japanese case,11
although the cow in the Japanese case was only 23 months old, and the cow in
this case was 64 months old.

The
fact that these strains were detected worldwide and in several breeds suggest
that there is no local or breed-dependent feature involved. It could be that the
WB techniques have become more specific within the past year in the detection of
minor differences in di-, mono-, and nonglycosylated molecular forms of PrPsc.
Infection of cattle by scrapie could also be considered since scrapie can be
transmitted by direct contact between animals or through environmental
contamination.13

In
conclusion, this Belgian case should be added to the list of atypical BSE
strains only very recently detected worldwide and may contribute to further
research studies about epidemiologic significance. Current continued research on
BSE would appear to reveal different BSE strains in analogy with the different
scrapie strains.

Bovine spongiform encephalopathy (BSE) is a prion disease with a fatal
neurodegenerative pathogenesis. It is characterized by the accumulation of an
abnormal protein (PrPres), formed posttranslationally from the normal isoform
(PrPc). Research in mice showed the existence of different sheep scrapie
strains. Scrapie strain discrimination is currently based upon biological typing
in a panel of inbred mice. However, no large scale studies of the molecular
features of PrPres have been reported for bovine BSE to date. Till now, the BSE
strain seemed to maintain constant biological and molecular properties even
after experimental or accidental passages into different species. Very recently,
variant forms of BSE have been reported in Japan, Italy and France. These forms
were characterized by atypical histopathological, immunohistochemical and/or
biochemical phenotype compared to the classical BSE strain. The present case
describes the first Belgian atypical BSE case. Since January 1st 2001, all
cattle older than 30 months is tested for TSE via a rapid test following EC
regulation 999/2001. Samples positive according to the ELISA screening are
further subjected to scrapie associated fibrils (SAF), histopathology,
immunohistochemistry and Western blot (WB) at the NRL. A positive ELISA sample
from a 64 month-old East-Flemish or Belgian white and red cow was presented at
the NRL for confirmation. The histopathology of the obex, pons and midbrain was
negative, immunohistochemistry and SAF were also negative. However, WB analysis
was positive with an electrophoretic profile different from that of a typical
BSE case. The band on the gel of the non-glycosylated form of PrPres of the
present case clearly showed a lower migratrion pattern compared to that of a
typical BSE case. For many years it was assumed that there was only one BSE
strain. Only very recently, reports of atypical BSE cases were announced in
Japan, Italy and France. The Japanese case describes a very young bull (23
months) negative on histopathology and immunohistochemistry and a WB
electrophoretic profile different from that of classical BSE. The Italians
observed two BSE affected cattle with a a different staining pattern on
immunohistochemistry, a difference in size and glycoform ratio of PrPres on WB
and a difference in regional distribution of lesions. The French two cases
showed variant molecular features with a different electrophoretic profile from
other BSE cases. The present case shows the most similarities with the Japanese
case (except for the age). The fact that these strains were detected worldwide
and in several breeds suggest that there is no local or breed dependent feature
involved. It could be that the WB techniques have become more specifique within
the last year or infection of cattle by scrapie could also be considered. In
conclusion, continued research on BSE reveals nowadays different BSE strains in
analogy with the different sheep scrapie strains. Atypical BSE cases may
question the significance and efficiency of the BSE epidemio-surveillance
protocol and the validation of the confirmatory tests.

Report on the assessment of the Geographical BSE-risk of BELGIUM July
2000

-
42 -

5.
CONCLUSION ON THE GEOGRAPHICAL BSE RISK

5.1
The current GBR

The
current geographical BSE-risk (GBR) level is III, i.e. BSE is confirmed in
domestic cattle (last and only case in 1997) at a lower level. However, the
observed incidence of clinical cases over the last 12 months (1 March 1999 to 29
February 2000) was 2.7 per 1 Million adult cattle. This figure is generated by a
passive surveillance system that is not able to identify all clinical
cases.

5.2
The expected development of the GBR

Assuming that measures in place continue to be appropriately implemented and no
new external challenge occurs, the GBR is expected to decrease over time.
However, this does not exclude that cattle infected by the BSE-agent in the past
(before 1998/99) may be identified as clinical cases in the foreseeable
future.

5.3
Recommendations for influencing the future GBR

Good implementation of the bans should be ensured.

In addition, expanding the surveillance system to target asymptomatic cattle in
risk sub-populations such as adult fallen stock and adult cattle presented for
emergency slaughter will allow verification of the current GBR-assessment and
monitoring its future trend.

Little is known about atypical BSE. The origin and natural routes of
transmission, if any, have yet to be determined. Almost all cases have been in
older cattle (usually > 8 years of age) that have shown little resemblance to
the clinic-pathological picture seen in classical disease. It has been suggested
that the disease may be sporadic or be caused by a genetic mutation, but no
convincing evidence has been found to support either of these ideas. The correct
answer will probably only come by study of the future annual incidence curves of
both types of disease. Regardless of the origin of atypical BSE, the possibility
of recycling the disease in cattle and other ruminants, as well as the potential
for transmission to humans, mandate a continuation of feed and specified-risk
materials (SRM) bans, together with diagnostic testing programs for some time to
come.

snip...

Naturally occurring cases of BSE in species other than cattle have been
very limited and have been linked to exposure to contaminated feed or infected
carcasses. The majority of cases originated in the UK and like BSE in cattle,
have declined with the implementation of feed controls. None of the exotic
animals were infected in the wild.

Experts who may be consulted: Linda A. Detwiler, DVM Clinical Professor
Department of Pathobiology and Population Medicine

College of Veterinary Medicine Mississippi State University 732-580-9391
Fax: 732-741-7751 ldetwiler@belle-terre.com

From 1998 a prospective surveillance study of Creutzfeldt-Jakob disease
(CJD) has been initiated in Belgium. In addition to epidemiological data,
information on cerebrospinal fluid biomarkers, prion protein gene and brain
neuropathology was collected. From 1-1-1998 to 31-12-2004, 188 patients were
referred to the surveillance system. In 85 patients a 'definite' diagnosis of
sporadic CJD (sCJD) could be made, whereas 26 patients remained 'probable'. We
further identified two unrelated patients with an E200K mutation, and two
patients with a seven octapeptide repeat insertion in one family. In one patient
a familial history was noted but genetic analysis was not performed. In 72
patients different final diagnoses were made, Alzheimer's disease being the most
frequent (N = 20). The demographic parameters of the Belgian population were
similar to those observed in the rest of Europe. We did notice a significantly
increased age-specific incidence (>6/106/ year) of sCJD patients between 70
and 90 years old in the period 2002-2004 compared to 1998-2001 and
retrospectively obtained data (1990-1997, p < 0.01). We undertook a detailed
clinical and biochemical analysis to investigate this increase but could not
identify any reason other than an increased vigilance for the diagnosis. In
conclusion, our study identified that in the past sCJD may have been
underestimated in patients over age 70 although these patients are both
clinically and neurobiochemically similar to the general sCJD phenotype.

To
date, 27 cases of L-BSE and 24 cases of H-BSE have been report­ed worldwide
(16), thus meaning that the prevalence of atypical BSE is considerably lower
than that of C-BSE. However, recent studies showed that L-BSE is easily
transmissible to transgenic mice expressing human (17,18) or bovine (19,20)
prion protein, as well as to non-human primates (21), with shorter incubation
periods than for the transmission of C-BSE to these animals.

***The virulent nature of L-BSE has stimulated new concern for human
public health since the transmis­sion of C-BSE to humans resulted in variant
Creutz­feldt-Jakob disease (vCJD) (4-7), a new emergent prion disease.

***The present data offer novel information on the tropism of the BASE
agent and highlight relevant public health issues. While the transmission
barrier for classical BSE is high in most species, BASE prions are readily
transmissible to a variety of mammals including non-human primates [11–13,35].
Accordingly, the possibility of spreading of BASE prions through skeletal muscle
to other species should be taken into account and evaluated in risk analysis
studies.

Background: Classical BSE is a world-wide prion disease in cattle, and
the classical BSE strain (BSE-C) has led to over 200 cases of clinical human
infection (variant CJD). Atypical BSE cases have been discovered in three
continents since 2004; they include the L-type (also named BASE), the H-type,
and the first reported case of naturally occurring BSE with mutated bovine PRNP
(termed BSE-M). The public health risks posed by atypical BSE were largely
undefined.

Objectives: To investigate these atypical BSE types in terms of their
transmissibility and phenotypes in humanized mice. Methods: Transgenic mice
expressing human PrP were inoculated with several classical (C-type) and
atypical (L-, H-, or Mtype) BSE isolates, and the transmission rate, incubation
time, characteristics and distribution of PrPSc, symptoms, and histopathology
were or will be examined and compared.

Results: Sixty percent of BASE-inoculated humanized mice became infected
with minimal spongiosis and an average incubation time of 20-22 months, whereas
only one of the C-type BSE-inoculated mice developed prion disease after more
than 2 years. Protease-resistant PrPSc in BASE-infected humanized Tg mouse
brains was biochemically different from bovine BASE or sCJD. PrPSc was also
detected in the spleen of 22% of BASE-infected humanized mice, but not in those
infected with sCJD. Secondary transmission of BASE in the humanized mice led to
a small reduction in incubation time. The atypical BSE-H strain is also
transmissible with distinct phenotypes in the humanized mice, but no BSE-M
transmission has been observed so far.

Discussion: Our results demonstrate that BASE is more virulent than
classical BSE, has a lymphotropic phenotype, and displays a modest transmission
barrier in our humanized mice. BSE-H is also transmissible in our humanized Tg
mice. The possibility of more than two atypical BSE strains will be
discussed.

***Moreover, L-BSE has been transmitted more easily to transgenic mice
overexpressing a human PrP [13,14] or to primates [15,16] than C-BSE.

***It has been suggested that some sporadic CJD subtypes in humans may
result from an exposure to the L-BSE agent.

***
Lending support to this hypothesis, pathological and biochemical similarities
have been observed between L-BSE and an sCJD subtype (MV genotype at codon 129
of PRNP) [17], and between L-BSE infected non-human primate and another sCJD
subtype (MM genotype) [15].

EFSA and the European Centre for Disease Prevention and Control (ECDC)
recently delivered a scientific opinion on any possible epidemiological or
molecular association between TSEs in animals and humans (EFSA Panel on
Biological Hazards (BIOHAZ) and ECDC, 2011). This opinion confirmed Classical
BSE prions as the only TSE agents demonstrated to be zoonotic so far

***
but the possibility that a small proportion of human cases so far classified as
"sporadic" CJD are of zoonotic origin could not be excluded.

The
TSE road map defining the evolution of European policy for protection against
prion diseases is based on a certain numbers of hypotheses some of which may
turn out to be erroneous. In particular, a form of BSE (called atypical Bovine
Spongiform Encephalopathy), recently identified by systematic testing in aged
cattle without clinical signs, may be the origin of classical BSE and thus
potentially constitute a reservoir, which may be impossible to eradicate if a
sporadic origin is confirmed.

***
Also, a link is suspected between atypical BSE and some apparently sporadic
cases of Creutzfeldt-Jakob disease in humans.

***
These atypical BSE cases constitute an unforeseen first threat that could
sharply modify the European approach to prion diseases.

RAPID ADVICE 17-2014 : Evaluation of the risk for public health of
casings in countries with a “negligible risk status for BSE” and on the risk of
modification of the list of specified risk materials (SRM) with regard to BSE

Although Bovine Spongiform Encephalopathy (BSE) is the cause of variant
Creutzfeldt Jakob disease (vCJD) in humans, the zoonotic potential of scrapie
prions remains unknown. Mice genetically engineered to overexpress the human
​prion protein (tgHu) have emerged as highly relevant models for gauging the
capacity of prions to transmit to humans. These models can propagate human
prions without any apparent transmission barrier and have been used used to
confirm the zoonotic ability of BSE. Here we show that a panel of sheep scrapie
prions transmit to several tgHu mice models with an efficiency comparable to
that of cattle BSE. The serial transmission of different scrapie isolates in
these mice led to the propagation of prions that are phenotypically identical to
those causing sporadic CJD (sCJD) in humans. These results demonstrate that
scrapie prions have a zoonotic potential and raise new questions about the
possible link between animal and human prions.

5.
A positive result from a chimpanzee challenged severly would likely create alarm
in some circles even if the result could not be interpreted for man. I have a
view that all these agents could be transmitted provided a large enough dose by
appropriate routes was given and the animals kept long enough. Until the
mechanisms of the species barrier are more clearly understood it might be best
to retain that hypothesis.

What if you can catch old-fashioned CJD by eating meat from a sheep
infected with scrapie?

28
Mar 01 Most doctors believe that sCJD is caused by a prion protein deforming by
chance into a killer. But Singeltary thinks otherwise. He is one of a number of
campaigners who say that some sCJD, like the variant CJD related to BSE, is
caused by eating meat from infected animals. Their suspicions have focused on
sheep carrying scrapie, a BSE-like disease that is widespread in flocks across
Europe and North America.

Now
scientists in France have stumbled across new evidence that adds weight to the
campaigners' fears. To their complete surprise, the researchers found that one
strain of scrapie causes the same brain damage in mice as sCJD.

"This means we cannot rule out that at least some sCJD may be caused by
some strains of scrapie," says team member Jean-Philippe Deslys of the French
Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses,
south-west of Paris. Hans Kretschmar of the University of Göttingen, who
coordinates CJD surveillance in Germany, is so concerned by the findings that he
now wants to trawl back through past sCJD cases to see if any might have been
caused by eating infected mutton or lamb...

2001

Suspect symptoms

What if you can catch old-fashioned CJD by eating meat from a sheep
infected with scrapie?

28
Mar 01

Like lambs to the slaughter

31
March 2001

by
Debora MacKenzie Magazine issue 2284.

FOUR years ago, Terry Singeltary watched his mother die horribly from a
degenerative brain disease. Doctors told him it was Alzheimer's, but Singeltary
was suspicious. The diagnosis didn't fit her violent symptoms, and he demanded
an autopsy. It showed she had died of sporadic Creutzfeldt-Jakob disease.

Most doctors believe that sCJD is caused by a prion protein deforming by
chance into a killer. But Singeltary thinks otherwise. He is one of a number of
campaigners who say that some sCJD, like the variant CJD related to BSE, is
caused by eating meat from infected animals. Their suspicions have focused on
sheep carrying scrapie, a BSE-like disease that is widespread in flocks across
Europe and North America.

Now
scientists in France have stumbled across new evidence that adds weight to the
campaigners' fears. To their complete surprise, the researchers found that one
strain of scrapie causes the same brain damage in mice as sCJD.

"This means we cannot rule out that at least some sCJD may be caused by
some strains of scrapie," says team member Jean-Philippe Deslys of the French
Atomic Energy Commission's medical research laboratory in Fontenay-aux-Roses,
south-west of Paris. Hans Kretschmar of the University of Göttingen, who
coordinates CJD surveillance in Germany, is so concerned by the findings that he
now wants to trawl back through past sCJD cases to see if any might have been
caused by eating infected mutton or lamb.

Scrapie has been around for centuries and until now there has been no
evidence that it poses a risk to human health. But if the French finding means
that scrapie can cause sCJD in people, countries around the world may have
overlooked a CJD crisis to rival that caused by BSE.

Deslys and colleagues were originally studying vCJD, not sCJD. They
injected the brains of macaque monkeys with brain from BSE cattle, and from
French and British vCJD patients. The brain damage and clinical symptoms in the
monkeys were the same for all three. Mice injected with the original sets of
brain tissue or with infected monkey brain also developed the same
symptoms.

As
a control experiment, the team also injected mice with brain tissue from people
and animals with other prion diseases: a French case of sCJD; a French patient
who caught sCJD from human-derived growth hormone; sheep with a French strain of
scrapie; and mice carrying a prion derived from an American scrapie strain. As
expected, they all affected the brain in a different way from BSE and vCJD. But
while the American strain of scrapie caused different damage from sCJD, the
French strain produced exactly the same pathology.

"The main evidence that scrapie does not affect humans has been
epidemiology," says Moira Bruce of the neuropathogenesis unit of the Institute
for Animal Health in Edinburgh, who was a member of the same team as Deslys.
"You see about the same incidence of the disease everywhere, whether or not
there are many sheep, and in countries such as New Zealand with no scrapie." In
the only previous comparisons of sCJD and scrapie in mice, Bruce found they were
dissimilar.

But
there are more than 20 strains of scrapie, and six of sCJD. "You would not
necessarily see a relationship between the two with epidemiology if only some
strains affect only some people," says Deslys. Bruce is cautious about the mouse
results, but agrees they require further investigation. Other trials of scrapie
and sCJD in mice, she says, are in progress.

People can have three different genetic variations of the human prion
protein, and each type of protein can fold up two different ways. Kretschmar has
found that these six combinations correspond to six clinical types of sCJD: each
type of normal prion produces a particular pathology when it spontaneously
deforms to produce sCJD.

But
if these proteins deform because of infection with a disease-causing prion, the
relationship between pathology and prion type should be different, as it is in
vCJD. "If we look at brain samples from sporadic CJD cases and find some that do
not fit the pattern," says Kretschmar, "that could mean they were caused by
infection."

There are 250 deaths per year from sCJD in the US, and a similar
incidence elsewhere. Singeltary and other US activists think that some of these
people died after eating contaminated meat or "nutritional" pills containing
dried animal brain. Governments will have a hard time facing activists like
Singeltary if it turns out that some sCJD isn't as spontaneous as doctors have
insisted.

Deslys's work on macaques also provides further proof that the human
disease vCJD is caused by BSE. And the experiments showed that vCJD is much more
virulent to primates than BSE, even when injected into the bloodstream rather
than the brain. This, says Deslys, means that there is an even bigger risk than
we thought that vCJD can be passed from one patient to another through
contaminated blood transfusions and surgical instruments.

what is CJD ? just ask USDA inc., and the OIE, they are still feeding
the public and the media industry fed junk science that is 30 years old.

why
doesn’t some of you try reading the facts, instead of rubber stamping everything
the USDA inc says.

sporadic CJD has now been linked to BSE aka mad cow disease, Scrapie,
and there is much concern now for CWD and risk factor for humans.

My
sincere condolences to the family and friends of the House Speaker Becky
Lockhart. I am deeply saddened hear this.

with that said, with great respect, I must ask each and every one of you
Politicians that are so deeply saddened to hear of this needless death of the
Honorable House Speaker Becky Lockhart, really, cry me a friggen river. I am
seriously going to ask you all this...I have been diplomatic for about 17 years
and it has got no where. people are still dying. so, are you all stupid or
what??? how many more need to die ??? how much is global trade of beef and other
meat products that are not tested for the TSE prion disease, how much and how
many bodies is this market worth?

***
Confirmed Variant Creutzfeldt-Jakob Disease (variant CJD) Case in Texas in June
2014 confirmed as USA case NOT European

the
patient had resided in Kuwait, Russia and Lebanon. The completed investigation
did not support the patient's having had extended travel to European countries,
including the United Kingdom, or travel to Saudi Arabia. The specific overseas
country where this patient’s infection occurred is less clear largely because
the investigation did not definitely link him to a country where other known
vCJD cases likely had been infected.